CASE REPORT Indonesian Journal of Neurosurgery (IJN) 2019, Volume 2, Number 3: 76-79 P-ISSN.2089-1180, E-ISSN.2302-2914 Jugular foramen lesion diagnostic dilemma – schwannoma or paraganglioma: A case report
Sachin Sambhaji Chemate1*, Joy Varghese2, Chandrasekar K2, Shankar Ganesh CV2, Mangaleswaran Balamurugan2
ABSTRACT
Introduction: Various anomalies occure in the jugular foramen. jugular foramen into the petrous temporal bone on the left side with The most common lesion within the jugular foramen is the prominent central flow voids. Paraganglioma was suspected and hypervascular glomus jugulare tumour followed by neurogenic Digital Subtraction Angiography was done which showed tumour tumors, predominantly schwannoma. Jugular foramen blush with arteriovenous fistula supplied by stylomastoid artery, schwannoma can also be vascularized and frequently mimics which is branch of occipital artery. Surprisingly, histopathology paraganglioma. after surgical resection revealed schwannoma. Case presentation: We present a case of a 36-year-old woman Conclusion: Prominent central vascularity and arteriovenous who presented with 8 to 10 months history of giddiness and fistula with arterial supply from stylomastoid branch of occipital vertigo, slowly progressive sensorineural hearing loss in left ear, artery can be an unusual presentation for jugular foramen gradually progressive left facial weakness (House Brackman grade schwannomas. We describe architecture of vascualrity of jugular III at the time of admission) and gait ataxia. A magnetic resonance schwannoma and identify stylomastoid branch of occipital artery imaging showed an avidly enhancing extra axial left cerebello- as its prime feeder. pontine angle space occupying lesion extending through the
Keywords: jugular foramen schwannoma, paraganglioma Cite This Article: Chemate, S.S., Varghese, J., Chandrasekar, K. Ganesh, S.C.V., Balamuragan, M. 2019. Jugular foramen lesion diagnostic dilemma – schwannoma or paraganglioma: A case report. Indonesian Journal of Neurosurgery 2(3): 76-79. DOI: 10.15562/ijn.v2i3.51
1Resident, Department of INTRODUCTION lesion like paraganglioma but very little information Neurosurgery, Apollo Hospitals, available in literature about the architecture of Chennai A diverse group of vascular anomalies originate in 2 its vascularity and no single feeding artery has Senior Consultant, Department 1-4 the jugular foramen. The most common tumour been identified for schwannoma in literature. The of Neurosurgery, Apollo Hospitals, within the jugular foramen is the hypervascular Chennai aim of this study is to identify the architecture of glomus jugulare tumour followed by neurogenic 5,6 vascularity and blood supply of the jugular foramen tumors, predominantly schwannoma. Less schwannoma. common lesions comprise meningioma, hemangiopericytoma, chondrosarcoma, and CASE PRESENTATION plasmacytoma. Metastases and malignant tumours arising in adjacent anatomic structures We present a case of a 36-year-old lady from (nasopharynx, parotid, and temporal bone) in northern part of India, known case of type II *Corresponding author: advanced stages may spread to the jugular foramen. diabetes. She presented with history of giddiness Sachin Sambhaji Chemate; Schwannomas involving the jugular foramen and vertigo since 10 months, gait imbalance since Resident, Department of are rare neoplasms and very few cases have 8 months with tendency to fall on left side, slowly Neurosurgery, Apollo Hospitals, been reported in the literature.7,8 Usually, it is progressive sensorineural hearing loss in left ear, Chennai; misdiagnosed as acoustic neuroma and the gradually progressive left facial weakness since 3 – 4 [email protected] diagnosis is made perioperatively.9,10 Schwannomas months, difficulty in swallowing liquids and change are relatively avascular but jugular foramen of voice since 1 month. schwannoma is known to be hypervascular On examination she was conscious and oriented. neoplasm. It has significant neurological morbidity There was no papilledema. She had signs of left and mortality.11-13 cerebellar hemisphere dysfunction with left VII Received: 2019-01-22 Paraganglioma is a vascular lesion with the nerve palsy (HB grade III), left ear sensorineural Accepted: 2019-02-03 ascending pharyngeal artery as a main feeder. The hearing loss, dysphagia for liquids and impaired Published: 2019-12-01 jugular foramen schwannoma is also hypervascular left gag reflex suggestive of left IX and X nerve
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impairment. Trapezius and sternocleidomastoid (DSA) which revealed a tumour blush in the muscle bulk was normal but weak on left side. region of jugular foramen which was suggestive Based on history and examinations, jugular of a hypervascular lesion (Figure 2). There was a foramen paraganglioma was suspected. Brain draining vein which was seen before appearance Magnetic Resonance Imaging (MRI) with contrast of tumor blush which was suggestive of an was done which showed an extraaxial mass arteriovenous fistula (Figure 3A and 3B). Only measuring 6.5 x 5.4 x 3.6 cm in left cerebellopontine one isolated feeder of arteriovenous fistula was angle space extending through the jugular foramen identified. In unsubtracted view, the same feeding into the petrous temporal bone on the left side vessel was seen from stylomastoid foramen (Figure which was hypointense on T1W and hyperintense 4B), hence stylomastoid artery (branch of occipital on T2W and FLAIR with heterogeneous contrast artery) was identified as the primary feeder of enhancement with salt and paper appearance lesion and the cause of arteriovenous shunting (Figure 1). (Figure 4A) in this case (and not by the ascending As paraganglioma was considered as first pharyngeal artery which is the known feeder of differential diagnosis and supported by brain MRI glomus jugulare). that was suggestive of a vascular lesion, the patient Arteriovenous shunting seen on DSA was more underwent Digital Subtraction Angiography suggestive of paraganglioma. Hence, we decided performed combined approach with preoperative embolization followed by craniotomy and excision of lesion. Preoperative embolization of feeding vessel was done. Post embolization, there was no tumor blush and fistula was completely obliterated. Patient underwent surgery post embolization. We used combined approach with translabyrynthine petrosectomy with left presigmoid and retrosigmoid craniotomy. Intraoperatively, tumour was highly vascular inspite of preoperative embolization. Complete excision of lesion was done. Figure 1. Brain MRI with GADO showing left cerebellopontine angle space Postoperatively patient had left side sensorineural occupying lesion extending through the jugular foramen into the hearing loss, left facial palsy, and dysphagia. She petrous temporal bone on the left side, showing heterogeneous was discharged on postoperative day 7th with Ryle’s contrast enhancement with salt and paper appearance. tube feeding. She was gradually started on speech and swallowing therapy. Histopathological examination showed fragments of spindle cell lesion composed of predominantly hypercellular areas with elongated bland nuclei and moderate eosinophilic cytoplasm (Antoni A) at places and hypocellular areas containing myxoid (Antoni B) were also seen. Verrocay bodies were evident. Few aggregates of fomy histiocytes and cystic changes were noted. Figure 2. DSA showing tumour blush with arteriovenous fistula. Focal lymphoid infiltrates were evident. Focal degenerative changes were also seen. Variable vascular density were noted composed of both thick and thin walled at foci showing proliferating vessels. This histomorphological features were consistent with schwannoma (WHO grade I) (Figure 5). At 6 month of follow up, brain MRI with contrast showed small residual lesion measuring 1.2 x 1.1 x 0.9 cm (Figure 6) which was managed with single fraction cyberknife therapy.
DISCUSSION Figure 3. A) Lateral view showing early filling of draining vein though a In present day, development of medical science fistula, B) Early feeling of draining vein even before appearance of has provided us better understanding of various tumor blush, suggestive of arteriovenous fistula. disease in cerebello-pontine angle and jugular
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palsy is not due to mechanical compression but it is because of ischemia due to shunting from stylomastoid branch of occipital artery. Schwannomas are typically described as avascular or hypovascular on angiography. We describe an unusual case of a jugular foramen schwannoma with blood supply from stylomastoid branch of occipital artery. To our knowledge this is the first case reported in the literature to talk about blood supply of jugular schwannoma, though we need more cases to support the evidence. Figure 4. A) Stylomastoid artery feeding the tumour, B) Arrow shows the artery in stylomastoid foramen in unsubstracted view. CONCLUSION Prominent central vascularity and arteriovenous fistula within the tumour can be an unusual presentation of jugular foramen schwannomas. We describe architecture of vascularity of jugular schwannoma and identify stylomastoid branch of occipital artery as its prime feeder. We also propose that the pathophysiology of VII nerve palsy is not Figure 5. HPE showing schwannoma (WHO grade I) due to mechanical compression but it is because of ischemia due to shunting from stylomastoid branch of occipital artery.
AUTHOR’S CONTRIBUTION All authors contribute equally in the collection, analysis, and interpretation of data. All authors have read and agreed to the final manuscript.
CONFLICT OF INTEREST There is no conflict of interest regarding this article. Figure 6. Post op scan at 6 month follow up showing small residual lesion FUNDING There is no sponsorship regarding this article. fossa.1-4 Whenever a vascular lesion was identified in jugular foramen, then paraganglioma was the REFERENCES: suspicion.5,6 Schwannomas elsewhere are relatively avascular or hypovascular on angiography. 1. Yousem DM, Grossman RI. Neuroradiology: The However, jugular foramen schwannomas are Requisites. 3rd ed. Philadelphia, Mosby; 2010. known to be hypervascular and very few cases have 2. Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR. The jugular foramen: a review of anatomy, masses, been reported in the literature.7-14 Lee M, et al13 and imaging characteristics. Radiographics. 1997; stated that, although hypervascular paraganglioma 17(5): 1123 – 1139. DOI: 10.1148/ is most common lesion in jugular foramen, radiographics.17.5.9308106. jugular schwannoma can present or mimic as 3. Vogl TJ, Bisdas S. Differential diagnosis of jugular paraganglioma. Ascending pharyngeal artery has foramen lesions. Skull Base. 2009; 19(1): 3 – 16. DOI: already been described and proven to be the prime 10.1055/s-0028-1103121. feeder of hypervascular paraganglioma.5-6 4. Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR, However, some case report argue that Pfeilsticker LN, Neto MC, et al. Jugular foramen ascending pharyngeal artery is also prime feeder tumors: diagnosis and treatment. Neurosurg Focus. 2004; 17(2): E5. DOI: 10.3171/foc.2004.17.2.5. of arteriovenous shunting seen in jugular foramen 8,9,11-13 5. Rao AB, Koeller KK, Adair CF. From the archives schwannoma. However, in our case, a of the AFIP. Paragangliomas of the head and stylomastoid branch of occipital artery is the actual neck: radiologic-pathologic correlation. Armed feeder of arteriovenous shunting (Figure 4A, 4B). Forces Institute of Pathology. Radiographics. 1999; We propose that the pathophysiology of VII nerve 19(6): 1605 – 1632. DOI: 10.1148/
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